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A new method to reconstruct the elastic modulus of soft tissue subjected to an external static compression is presented. In this approach the Newton-Raphson method is used to vary a finite element (FE) model of the elasticity equations to fit, in a least squared sense, a set of axial tissue displacement fields estimated using a correlation technique applied to ultrasound signals. The ill-conditioning of the Hessian matrix is eliminated using the Tikhonov regularization technique. This regularization provides a compromise between fidelity to the observed data and a priori information of the solution. Using an echographic image formation model, it is shown that the method converges within a few iterations (8-10) and that strain images artifacts which are common in elastography are significantly reduced after the resolution of the inverse problem.

Abstract Background Gaucher disease (GD) is the most frequent lysosomal storage disorder; type 1 is by far the most common form. It is characterized by variability in age of onset, clinical signs and progression. It is usually diagnosed in the first or second decade of life with the appearance of bone pains, splenomegaly and thrombocytopenia, but the disease may be diagnosed at any age between 1 and 73 years. In the present study, we report 3 cases with late onset of GD in whom the disease was a surprise finding including one patient with Parkinson disease. This late onset is described as an adult form of Gaucher disease. Findings Molecular investigation showed mutational homogeneity in Tunisian adult patients suffering from GD. Indeed, all patients carry the p.N370S mutation: two patients at a homozygous state and one patient at compound heterozygous state. Conclusion The p.N370S mutation presents a large variability in the onset of the disease and its clinical manifestation supporting the view that GD should be considered as a continuum phenotype rather than a predefined classification.

Considering the current growing interest in new and improved enzymes for use in a variety of applications, the present study aimed to characterize a novel detergent-stable serine alkaline protease from the extremophilic actinobacterium Microbacterium metallidurans TL13 (MmSP) using a combined in silico and experimental approach. The MmSP showed a close phylogenetic relationship with high molecular weight S8 peptidases of Microbacterium species. Moreover, its physical and chemical parameters computed using Expasy's ProtParam tool revealed that MmSP is hydrophilic, halophilic and thermo-alkali stable. 3D structure modelling and functional prediction of TL13 serine protease resulted in the detection of five characteristic domains: [catalytic subtilase domain, fibronectin (Fn) type-III domain, peptidase inhibitor I9, protease-associated (PA) domain and bacterial Ig-like domain (group 3)], as well as the three amino acid residues [aspartate (D182), histidine (H272) and serine (S604)] in the catalytic subtilase domain. The extremophilic strain TL13 was tested for protease production using agricultural wastes/by-products as carbon substrates. Maximum enzyme activity (390 U/gds) was obtained at 8th day fermentation on potato peel medium. Extracellular extract was concentrated and partially purified using ammonium sulfate precipitation methodology (1.58 folds purification fold). The optimal pH, temperature and salinity of MmSP were 9, 60 °C and 1 M NaCl, respectively. The MmSP protease showed broad pH stability, thermal stability, salt tolerance and detergent compatibility. In order to achieve the maximum stain removal efficacy by the TL 13 serine protease, the operation conditions were optimized using a Box-Behnken Design (BBD) with four variables, namely, time (15-75 min), temperature (30-60 °C), MmSP enzyme concentration (5-10 U/mL) and pH (7-11). The maximum stain removal yield (95 ± 4%) obtained under the optimal enzymatic operation conditions (treatment with 7.5 U/mL of MmSP during 30 min at 32 °C and pH9) was in good agreement with the value predicted by the regression model (98 ± %), which prove the validity of the fitted model. In conclusion, MmSP appears to be a good candidate for industrial applications, particularly in laundry detergent formulations, due to its high hydrophilicity, alkali-halo-stability, detergent compatibility and stain removal efficiency., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest in the publication., (© King Abdulaziz City for Science and Technology 2024. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Aims: Evaluation of left and right ventricular (RV) longitudinal systolic function may enhance risk stratification following aortic valve replacement (AVR). The study objective was to evaluate the changes in left and RV longitudinal systolic function and RV-pulmonary artery (RV-PA) coupling from baseline to 30 days and 1 year after AVR., Methods and Results: Left ventricular (LV) longitudinal strain (LS), tricuspid annulus plane systolic excursion (TAPSE), and RV-PA coupling were evaluated in patients from the PARTNER 2A surgical AVR (SAVR) arm (n = 985) and from the PARTNER 2 SAPIEN 3 registry (n = 719). TAPSE and RV-PA coupling decreased significantly following SAVR, but remained stable following TAVR. Lower LV LS, TAPSE, or RV-PA coupling at baseline was associated with increased risk of the composite of death, hospitalization, and stroke at 5 years [adjusted hazard ratios (HRs) for LV LS < 15%: 1.24, 95% confidence interval (CI) 1.05-1.45, P = 0.001; TAPSE < 14 mm: 1.44, 95% CI 1.21-1.73, P < 0.001; RV-PA coupling < 0.55 mm/mmHg: 1.32, 95% CI 1.07-1.63, P = 0.011]. Reduced TAPSE at baseline was the most powerful predictor of the composite endpoint at 5 years. Patients with LV ejection fraction <50% at baseline had increased risk of the primary endpoint with SAVR (HR: 1.34, 95% CI 1.08-1.68, P = 0.009) but not with TAVR (HR: 1.12, 95% CI 0.88-1.42). Lower RV-PA coupling at 30 days showed the strongest association with cardiac mortality., Conclusion: SAVR but not TAVR was associated with a marked deterioration in RV longitudinal systolic function and RV-PA coupling. Lower TAPSE and RV-PA coupling at 30 days were associated with inferior clinical outcomes at 5 years. In patients with LVEF < 50%, TAVR was associated with superior 5-year outcomes., Competing Interests: Conflict of interest: J.T. is consultant for Abbott, Philips Healthcare, and General Electric. R.T.H. reports speaker fees from Abbott Structural, Baylis Medical, Edwards Lifesciences, Medtronic, and Philips Healthcare; she has institutional consulting contracts for which she receives no direct compensation with Abbott Structural, Edwards Lifesciences, Medtronic, and Novartis; she is Chief Scientific Officer for the Echocardiography Core Laboratory at the Cardiovascular Research Foundation for multiple industry-sponsored tricuspid valve trials, for which she receives no direct industry compensation. M.-A.C. has research grant with Medtronic and core laboratory contract with Edwards Lifesciences without direct compensation. W.J. is consultant for Boston Scientific and BridgeBio. F.M.A. and N.J.W. have institutional research grants as directors of an academic core lab from Edwards, Abbott, Medtronic, Boston Scientific, Biotronik, Corcyn, and Foldax. They have no personal disclosures. N.A. received speakers fees from Abbott Vascular, GE Healthcare, and Philips ultrasound and research grants from Alnylam and Pfizer. H.C.H. reports institutional research funding from Abbott, Boston Scientific, Edwards Lifesciences, Highlife, Medtronic, and WL Gore; consulting fees from Edwards Lifesciences, Medtronic, Wells Fargo, and WL Gore; and equity in Holistick Medical and Micro Interventional Devices. M.J.M. has served as a Co-Pi for clinical trials for Abbott and Edwards Lifesciences and as Study Chair for a trial for Medtronic. All roles were uncompensated. V.T. is consultant or researcher for Abbott Vascular, Boston Scientific, CryoLife, Edwards Lifesciences, Medtronic, and Shockwave. The other authors have nothing to disclose., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Objective: Biclonal gammopathies (BGs) are rare situations characterized by the production of 2 monoclonal proteins. There are no available data on BGs in North Africa. We aimed to estimate the prevalence of BGs in our population and describe their clinical and laboratory features., Methods: We conducted a 31-year retrospective study including patients with persistent double monoclonal bands based on the results of immunofixation/immunoelectrophoresis., Results: A total of 35 patients with available clinical data (sex ratio, M/F = 1.53; mean age, 70 ± 10.87 years [range, 45-90 years]) were included. The main associated conditions were multiple myeloma (MM) (40%), BG of undetermined significance (BGUS) (34%), and lymphoproliferative diseases (23%). Only one-third of the patients had 2 monoclonal spikes on serum protein electrophoresis. The most common paraprotein combinations were immunoglobulin (Ig)G-IgG (25%) and IgG-IgA (23%) with different light chains in one-half of the cases. The mean follow-up was 25.6 months (median, 12 months). No BGUS evolved into a malignant disease., Conclusion: BGs are rare in clinical laboratory routine but must be accurately identified by the pathologist. Our cohort is characterized by a high prevalence of BGUS compared with MM., (© The Author(s) 2023. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction: Langerhans cell sarcoma (LCS) is a very rare malignant tumor of Langerhans cells that may metastasize to many organs. The diagnosis of this tumor is difficult and its prognosis is poor., Aim: To report the difficulty to diagnose LCS, and discuss therapeutic management of this rare entity., Case Presentation: We report a case of LCS in a 52-year-old man who presented with an axillar lymphadenopathy. The diagnosis of nodular sclerosis type Hodgkin's disease was established after histologic examination. The patient was treated with chemotherapy (ABVD regimen: Doxorubicin, Bleomycin, Vinblastine, Dacarbazine) and radiotherapy with a partial response. However, disease recurrence was observed and histological analysis confirmed the diagnosis of Langerhans cell sarcoma. A revision of the initial histological examination concluded to the diagnosis of sarcoma from the beginning. We chose the ESHAP (Etoposide, Methylprednisolone, Aracytine, Cisplatin) regimen and clinical improvement of LCS was obtained after 2 cycles but the patient had a fatal outcome and died by disease progression., Conclusion: Because of its rarity, diagnosis is difficult and an optimal treatment strategy for this disease has not yet been identified. Polychemotherapy can be an effective modality for the treatment of LCS.

Multiple sclerosis (MS) is one of the most serious neurological diseases. It is the most frequent reason of non-traumatic disability among young adults. MS is an autoimmune disease wherein the central nervous system wrongly destructs the myelin sheath surrounding and protecting axons of nerve cells of the brain and the spinal cord which results in presence of lesions called plaques. The damage of myelin sheath alters the normal transmission of nerve flow at the plaques level, consequently, a loss of communication between the brain and other organs. The consequence of this poor transmission of nerve impulses is the occurrence of various neurological symptoms. MS lesions cause mobility, vision, cognitive, and memory disorders. Indeed, early detection of lesions provides an accurate MS diagnosis. Consequently, and with the adequate treatment, clinicians will be able to deal effectively with the disease and reduce the number of relapses. Therefore, the use of magnetic resonance imaging (MRI) is primordial which is proven as the relevant imaging tool for early diagnosis of MS patients. But, low contrast MRI images can hide important objects in the image such lesions. In this paper, we propose a new automated contrast enhancement (CE) method to ameliorate the low contrast of MRI images for a better enhancement of MS lesions. This step is very important as it helps radiologists in confirming their diagnosis. The developed algorithm called BDS is based on Brightness Preserving Dynamic Fuzzy Histogram Equalization (BPDFHE) and Singular Value Decomposition with Discrete Wavelet Transform (SVD-DWT) techniques. BDS is dedicated to improve the low quality of MRI images with preservation of the brightness level and the edge details from degradation and without added artifacts or noise. These features are essential in CE approaches for a better lesion recognition. A modified version of BDS called MBDS is also implemented in the second part of this paper wherein we have proposed a new method for computing the correction factor. Indeed, with the use of the new correction factor, the entropy has been increased and the contrast is greatly enhanced. MBDS is specially dedicated for very low contrast MRI images. The experimental results proved the effectiveness of developed methods in improving low contrast of MRI images with preservation of brightness level and edge information. Moreover, performances of both proposed BDS and MBDS algorithms exceeded conventional CE methods., (© 2022. The Author(s) under exclusive licence to Society for Imaging Informatics in Medicine.)

Objectives: The aim of this study was to explore the literature in order to assess systematically the association between amelogenesis imperfecta (AI) and caries development and to evaluate the DMF index among AI patients. Basic Research Design . PubMed was used to explore the database Medline. The key words used were "Amelogenesis Imperfecta" [Mesh], "Dental Caries" [Mesh], "Tooth Loss" [Mesh], "DMF Index" [Mesh], and "Dental Restoration, Permanent" [Mesh]. Moreover, an ad hoc search was performed in order to make the study as exhaustive as possible., Results: Fifty-five articles were retained. The total number of patients gathered was 499. A percentage of 68.8% of the articles dealt with cases with a relatively low dental caries process, 20.8% dealt with cases in which the dental caries process was relatively moderate, and 10.4% dealt with cases in which the dental caries process was severe. Teeth extraction due to dental caries was mentioned in 10 articles. Eleven articles, concerning 53 patients, mentioned dental fillings. Four patients did not have dental filling due to dental caries. DMF index was very low in 2 articles and low-to-high in 3 articles., Conclusion: Low dental caries susceptibility with AI patients was noticed in this study. A possible factor could be the lack of proximal contacts and elimination of fissures through enamel loss. The lack of dental caries susceptibility was also explained by the microbacterial specificity of hypoplastic AI patients. Moreover, it was also noted that the prevalence of dental caries among AI patients depends on sociodemographic change., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Farah Kallel et al.)

Resting state functional MRI (rs-fMRI) is an imaging technique that allows the spontaneous activity of the brain to be measured. Measures of functional connectivity highly depend on the quality of the BOLD signal data processing. In this study, our aim was to study the influence of preprocessing steps and their order of application on small-world topology and their efficiency in resting state fMRI data analysis using graph theory. We applied the most standard preprocessing steps: slice-timing, realign, smoothing, filtering, and the tCompCor method. In particular, we were interested in how preprocessing can retain the small-world economic properties and how to maximize the local and global efficiency of a network while minimizing the cost. Tests that we conducted in 54 healthy subjects showed that the choice and ordering of preprocessing steps impacted the graph measures. We found that the csr (where we applied realignment, smoothing, and tCompCor as a final step) and the scr (where we applied realignment, tCompCor and smoothing as a final step) strategies had the highest mean values of global efficiency (eg) . Furthermore, we found that the fscr strategy (where we applied realignment, tCompCor, smoothing, and filtering as a final step), had the highest mean local efficiency (el) values. These results confirm that the graph theory measures of functional connectivity depend on the ordering of the processing steps, with the best results being obtained using smoothing and tCompCor as the final steps for global efficiency with additional filtering for local efficiency.

Background: Nonsurgical bleeding is a frequent complication of continuous-flow left ventricular assist device (LVAD) support. Abnormal von Willebrand factor (vWF) metabolism plays a major role. However, the relationship between LVAD speed and vWF degradation is unknown. Recent evidence has demonstrated that supraphysiologic shear stress from continuous-flow LVADs accelerates vWF degradation and causes an acquired vWF deficiency and bleeding. To manage LVAD-associated bleeding, it has been proposed that reduced LVAD speed may decrease shear stress and thereby reduce pathologic vWF metabolism. However, there are little published data to support this clinical practice. We tested the hypothesis that reduced continuous-flow LVAD speed decreases vWF degradation., Methods: Whole blood was collected from patients before and after the implantation of a HeartMate II continuous-flow LVAD (n = 10) to quantify in vivo vWF degradation. In parallel, to evaluate the relationship between LVAD rpm and vWF degradation, whole blood was collected from human donors (n = 30). Single-donor units of blood circulated in an ex vivo HeartMate II mock circulatory loop for 12 hours at 11,400, 10,000, or 8600 rpm (n = 10/each rpm group). vWF multimers and degradation fragments were characterized with electrophoresis and immunoblot analysis. Paired Student t tests were performed within each group. ANOVA with Tukey post hoc test was performed across groups., Results: In patients, LVAD support reduced large vWF multimers and significantly (P < .05) increased vWF degradation fragments. The profile of vWF degradation was nearly identical between LVAD patients and blood circulated in the LVAD mock circulatory loop. At 11,400, 10,000, and 8600 rpm, decreased large vWF multimers and significantly increased vWF degradation fragments were noted. vWF degradation fragments were not statistically different across the 3 rpm groups or versus LVAD patients, which suggested that LVAD rpm did not influence vWF degradation., Conclusions: Reduced LVAD speed (within the clinical operational range) did not significantly decrease vWF degradation in a mock circulatory loop with human blood. During bleeding events, reduced LVAD speed, itself, may not diminish vWF degradation., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: The differentiated thyroid cancers have a good prognosis unless the presence of metastasis. These distant metastases, especially in bone, are a major cause of impaired quality of life and death requiring intensive management. The aim of our work was to study the patients' data, the disease characteristics and to analyze the therapeutic management of these patients., Results: This study concerned a cohort of 21 patients treated for differentiated thyroid cancer during the period from 1995 to 2011. Eighteen of our patients were aged over 45 years. A majority of them had follicular carcinoma. Bone metastases were often multiple and located at the axial skeleton. They were associated with other types of metastases, especially lung metastasis. A majority of patients received 131I treatment, following surgery or external beam radiotherapy for a palliative purpose. Overall survival was 65% at 5 years and 49% at 10 years. A long-term survival was achieved in 10% of the patients benefiting from a multidisciplinary care adapted to each case., Conclusion: Bone metastases often have a pejorative turning in the natural history of differentiated thyroid cancers. The right selection of individuals with better prognosis, for whom more aggressive curative treatment was indicated, requires a better understanding of the features of bone involvement.

The effects of high intensity focused ultrasound (HIFU)-induced continuously varying thermal gradients on sound ray propagation were modeled theoretically. This modeling was based on Fermat's variational principle of least time for rays propagating in a continuously varying thermal gradient described by a radially symmetric heat equation. Such thermal lenses dynamically affect HIFU beam focusing, and simultaneously create ultrasonic geometric and intensity distortions and artifacts in monitoring devices. Techniques which are based upon ultrasonic cross-correlation methods, such as elastography and two-dimensional temperature estimation, also suffer distortion effects and generate artifacts.